Pyrimidine 5'-nucleotidase and oxidative damage in red blood cells transfused to beta-thalassemic children.
Pyrimidine 5' nucleotidase (P5'N) acquired deficiency has been found in several hematologic disorders including beta-thalassemia. Our previous studies suggested that the aldehydes produced during membrane lipid peroxidation could play a role in P5'N inactivation in thalassemia. To evaluate the effects of the thalassemic "environment" on transfused red blood cells, we tested P5'N, pyruvate kinase (PK), glucose 6-phosphate dehydrogenase (G-6PD) activity, creatine content, reduced glutathione (GSH) levels and the hexose monophosphate shunt (HMS) in the red cells of homozygous transfusion-dependent thalassemic children, immediately following and again one month after transfusion. In red cells aged in thalassemic plasma, P5'N activity, creatine level, GSH stability and stimulated HMS flux were significantly decreased. These results fit in with the presence in thalassemic plasma of molecules interfering with antioxidant red cell defenses. Normal red cells incubated in thalassemic plasma display a significant stimulation of the basal HMS (p less than 0.01). Transfused red cell metabolic alterations could be explained by the plasma pro-oxidant activity and may contribute to reducing red cell survival in the host plasma.
David
O
O
Istituto di Clinica Pediatrica, Università, Torino, Italy.
Sacchetti
L
L
Vota
M G
MG
Comino
L
L
Perugini
L
L
Pescarmona
G P
GP
D015720:5'-Nucleotidase / Q000097:blood* / Q000172:deficiency; D001803:Blood Transfusion; D002648:Child; D002675:Child, Preschool; D003401:Creatine / Q000097:blood; D004905:Erythrocyte Aging; D017707:Erythrocyte Transfusion; D004912:Erythrocytes / Q000201:enzymology*; D005954:Glucosephosphate Dehydrogenase / Q000097:blood; D005978:Glutathione / Q000097:blood; D006801:Humans; D015227:Lipid Peroxidation; D010084:Oxidation-Reduction; D010427:Pentose Phosphate Pathway; D011770:Pyruvate Kinase / Q000097:blood; D013789:Thalassemia / Q000097:blood* / Q000628:therapy
D005954:Glucosephosphate Dehydrogenase; D011770:Pyruvate Kinase; D015720:5'-Nucleotidase; D005978:Glutathione; D003401:Creatine